Competency based curriculum for cardiovascular magnetic resonance: A position statement of the Society for Cardiovascular Magnetic Resonance.

This position statement guides cardiovascular magnetic resonance (CMR) imaging program directors and learners on the key competencies required for Level II and III CMR practitioners, whether trainees come from a radiology or cardiology background. This document is built upon existing curricula and was created and vetted by an international panel of cardiologists and radiologists on behalf of the Society for Cardiovascular Magnetic Resonance (SCMR).

The mass that should not be there: Multi-modality imaging shapes diagnostic reasoning.

A rare case of pulmonary artery fibroelastoma that demonstrates the importance of multimodality imaging and serial scans in reducing diagnostic uncertainty.

Cardiac rhabdomyoma: an uncommon culprit in sudden infant death.

Sudden death in infants remains a common and poorly understood cause of childhood mortality in the USA. Pediatric cardiac tumors, although rare, may underlie some cases of unexplained sudden infant death. Autopsy is a crucial diagnostic step in these cases, as both gross and microscopic examination of the heart may uncover occult cardiac tumors. Rhabdomyomas are the most common cardiac tumors in childhood and may result in arrythmia and sudden death. We present a case of sudden death in a healthy 5-month-old infant which initially appeared "SIDS-like" until thorough histological analysis revealed an underlying cardiac rhabdomyoma. The case is of particular importance in that the gross examination of the heart was considered completely normal, and the tumor only involved certain portions of the heart microscopically. Had a single random section of myocardium been the only heart section examined microscopically, the diagnosis might have been missed. This case emphasizes the importance of thorough microscopic examination in infant cases, especially in cases where the heart appears grossly normal.

Malignant Primary and Metastatic Cardiac Tumors: A Single-Center 27-Year Case Review.

BACKGROUND: Malignant primary cardiac tumors are exceedingly rare, and despite surgical exeresis or chemotherapy, their prognosis remains poor. Cardiac invasion by metastatic tumors, while more common, also entails an unsatisfactory outcome. This study aimed to review patients diagnosed with malignant primary and secondary cardiac tumors in a tertiary center between 1995 and 2022. METHODS: Clinical data, echocardiographic, computed tomography, and magnetic resonance assessments of tumor location and morphology, histology, treatment, and survival were retrospectively analyzed. RESULTS: Sixty malignant cardiac tumors were diagnosed: 17 primary (A) and 43 metastatic (B) tumors. A: the most common types were angiosarcoma (41%), undifferentiated sarcoma (23%), and fibrosarcoma (18%). Patients with primary tumors were younger than patients with metastatic tumors (41 ± 13 years vs. 57 ± 18 years, p = 0.001), with no significant gender difference. The most frequent presentations were heart failure (59%) and arrhythmia (23%). The most prevalent tumor location was the right heart chambers (71%), mostly in the right atrium (35%). 47% were submitted to tumor resection, and 29% received chemotherapy. The mortality rate was 82% with a median survival of 6.0 (interquartile range: 1.0-11.8) months after diagnosis (minimum of 12 days and maximum of 19 years). One patient with fibrosarcoma underwent heart transplantation and was still alive and well after 19 years. B: regarding metastatic cardiac invasion, the most common primary tumor sites were lung carcinomas (38%), thymomas (17%), and lymphomas (14%). Presentation with pericardial effusion was common (33%). The mortality rate was 72%, with a median survival of 3.6 (1.0-13.4) months (minimum of 7 days, maximum of 5 years). CONCLUSION: Diagnosis of metastatic cardiac tumors was more common than that of malignant primary tumors, both with a dismal prognosis. When radical exeresis is not possible, heart transplantation can be an option with a favorable outcome in carefully selected patients with sarcomas.

Echocardiographic Evaluation of Cardiac Masses.

PURPOSE OF REVIEW: Cardiac masses encompass a broad range of etiologies and are often initially revealed by echocardiography. The differential may change depending on the location of the mass and patients' medical history or presentation. It is important for clinicians to be aware of subtle visual characteristics on echocardiography in order to correctly diagnose the pathology. METHODS: Patients who underwent transthoracic echocardiography and were found to have one or more cardiac masses between January 1, 2020, and May 15, 2023, were reviewed. Their demographic data, clinical presentation, medical history, imaging, and follow-up information were collected from hospital electronic medical records, de-identified, and used to complete this review paper. A detailed review of cardiac masses divided by cardiac chamber accompanied by real-world echocardiographic images from patients in a large inner city public hospital. We hope that this systematic review of cardiac masses with real-world echocardiographic images will help clinicians note subtle echocardiographic characteristics to aid in the diagnosis and treatment of cardiac masses.

Magnetic Resonance Imaging in the Study of Cardiac Masses: A Case Series.

Cardiac masses are currently studied using multimodality imaging. For diagnosis, different imaging techniques that can provide complementary information are used. Cardiac magnetic resonance imaging (MRI) has become a fundamental tool for this type of pathology owing to its ability to provide tissue characterization, spatial accuracy, and the anatomic relationships of the different structures. This study presents a series of four clinical cases with an initial diagnosis of a cardiac mass. All cases were evaluated at a single center, and patients were aged 57 to 72 years. An etiological study was conducted on all patients using different imaging techniques, including MRI. This study describes the diagnostic and therapeutic procedures of the four cases, which included two intracardiac metastases and two benign tumors. Cardiac MRI was decisive in the diagnostic process, determining the clinical decision-making in all four cases. Cardiac MRI has emerged as a pivotal technique in the diagnosis of cardiac masses. It can provide a highly accurate histological diagnosis without the need for invasive techniques.

Cardiac tumors-sex-related characteristics and outcomes after surgical resection.

OBJECTIVES: Cardiac tumors represent a rare and heterogeneous pathological entity, with a cumulative incidence of up to 0.02%. Gender was previously reported to influence outcomes after tumor surgery. This study aimed to investigate for gender-related differences in outcomes after cardiac surgery. METHODS: Between 2009 and 2021, 95 male and 88 female patients underwent surgery for tumor extirpation in our center. Preoperative baseline characteristics, intraoperative data, and long-term survival were analyzed. The diagnosis was confirmed postoperatively by (immune-)histopathological analysis. RESULTS: There were no significant differences in baseline characteristics and survival. Myxoma was the most common tumor type overall and was more diagnosed in women (n = 36 vs. n = 62, p ≤ 0.001). Sarcoma was the most common malignant tumor type (n = 5). Tumor location at the atrial septum was more likely in women (n = 26 vs. n = 16, p = 0.041), whereas ventricular localization was more common in male patients (n = 20 vs. n = 7, p = 0.001). Minimally invasive tumor extirpation was significantly more often performed in women, and in-hospital stay was shorter in female patients. CONCLUSION: The localization and dignity of cardiac tumors differ between genders, not affecting survival. Surgical tumor extirpation remains the gold standard of treatment for cardiac tumors in both genders as it is highly effective and associated with good long-term survivorship.

Diagnostic challenges in primary cardiac lymphoma, the opportunity of 18F-FDG PET/CT integrated with contrast-enhanced CT.

BACKGROUND: The purpose of this study was to retrospectively evaluate the value of 18F-FDG PET/CT integrated with contrast-enhanced CT (CECT) in the differential diagnosis of primary cardiac lymphomas (PCLs) and primary cardiac angiosarcomas (PCAs). METHODS: Clinical and imaging data of patients with PCLs and PCAs were collected. All patients underwent preoperative 18F-FDG PET/CT and thoracic CECT. The enhancement pattern and tumor morphology were analyzed using CECT images. The intensity- and volume-based PET parameters of cardiac lesions were analyzed. The performance characteristics of all parameters were assessed. RESULTS: Nine patients with PCL and eight patients with PCA were analyzed. There were significant differences in SUVmax (t = 3.790, P = .002), SUVmean (t = 4.273, P = .001), metabolic tumor volume (U = 13.00, P = .027), tumor-to-liver ratio (U = 10.00, P = .011), and total lesion glycolysis (U = 4.0, P = .001) between PCLs and PC18As. There were significant differences in the enhancement pattern of tumors (P = .002) and tumor morphology (P = .015). The combination of F-FDG PET/CT and CECT improved the diagnostic accuracy, and the combination cutoff (SUVmean > 5.17) could reach 100%, but the difference was not statistically significant (P > .05). CONCLUSION: The intensity- and volume-based PET parameters of PCL were significantly higher than those of PCA. The enhancement pattern and tumor morphology were also different. According to these characteristics, the two most common types of primary cardiac malignancies can be differentiated.

Strategies to rare primary cardiac lipomas in the left ventricle in a patient: case report.

BACKGROUND: Primary cardiac tumors are rare in all age groups and are usually benign. Symptoms are usually related to tumor size, location, invasiveness, number, and growth rate. While histologically benign, cardiac arrest may be caused by blocked inflow or outflow or malignant ventricular arrhythmia. Surgical resection of left ventricular tumors, especially those involving the outflow tract, is challenging. CASE PRESENTATION: Herein, we present a rare case of an asymptomatic, 39-year-old woman who was referred to our cardiovascular department for a huge left ventricular cardiac mass incidentally discovered during the physical examination. Images showed a huge mass that quasi-circular low-density focus with a clear boundary and regular shape in the left ventricular cavity and fortunately had no significant effect on the peripheral valves and hemodynamics. CONCLUSIONS: This illustrative report highlights the exact surgical management of a cardiac tumor depends largely on the site and extent of the mass. Mechanical compromise and not the neoplastic potential should be considered. A conservative approach and follow-up regularly are advocated to ensure that the patient gets the best diagnosis and treatment, however, surgery is indicated only for severely symptomatic patients with hemodynamic compromise.

Atrial septal defect with a rare occupying lesion in heart.

BACKGROUND: Cardiac epicardium hemangiomas are exceedingly rare; however, they can cause significant hemodynamic impairment and large pericardial effusions. On rare occasion, cardiac tumors coexist with malformations of the heart. CASE PRESENTATION: We present the case of a 10-month-old female infant with a rare cardiac surface hemangioma coexisting with malformations of the heart. It revealed an atrial septal defect (ASD) coexisting with an abnormal occupying lesion with high echogenicity. It was 35*12*9 mm in size and was found in the anterior atrioventricular junction to the posterior atrioventricular junction at the bottom of the ventricular septum by transthoracic echocardiography. We performed surgical treatment of the atrial septal defect and performed biopsy with the occupying lesion. The histopathological examination reported a benign tumor as hemangioma. As far as we know, this is the first case in which cardiac surface hemangioma was found to coexist with an atrial septal defect. CONCLUSIONS: Cardiac epicardium hemangiomas is a rare solid tumor of the heart. If the mass is impossible to resect and does not cause hemodynamic impairment, only mass biopsy is possible.

Unusual diagnosis of feline cardiac lymphoma using cardiac needle biopsy.

BACKGROUND: Cardiac tumors in cats are relatively rare, with lymphoma accounting for more than half of all cases. However, feline cardiac lymphoma is often diagnosed post-mortem, and it is difficult to diagnose while the cat is still alive. It is the first report of a direct, rather than estimative, diagnosis with cardiac needle biopsy of a living cat with cardiac lymphoma. CASE PRESENTATION: A 3-year-old domestic short-haired male cat experienced loss of energy and loss of appetite. Thoracic radiography and transthoracic echocardiography showed cardiomegaly with slight pleural effusion and cardiac tamponade due to pericardial effusion, respectively. In addition, partial hyperechoic and hypertrophy of the papillary muscle and myocardium were observed. Blood test showed an increase in cardiac troponin I levels. Pericardial fluid, removed by pericardiocentesis, was analyzed; however, the cause could not be determined. With the owner's consent, pericardiectomy performed under thoracotomy revealed a discolored myocardium. Cardiac needle biopsy was performed with a 25G needle, and a large number of large atypical lymphocytes were collected; therefore, a direct diagnosis of cardiac lymphoma was made. Pathological examination of the pericardium diagnosed at a later date revealed T-cell large cell lymphoma. The cat underwent chemotherapy followed by temporary remission but died 60 days after the diagnosis. Postmortem, two-dimensional speckle-tracking echocardiography (data when alive) revealed an abnormal left ventricular myocardial deformation, which corresponded to the site of cardiac needle biopsy. CONCLUSIONS: This rare case demonstrates that cardiac lymphoma should be added to the differential diagnosis in cats with myocardial hypertrophy and that the diagnosis can be made directly by thoracotomy and cardiac needle biopsy. In addition, the measurement of cardiac troponin I levels and local deformation analysis of the myocardium by two-dimensional speckle-tracking echocardiography may be useful in the diagnosis of cardiac tumors.

Primary malignant cardiac tumors: Sex-related therapy and multidisciplinary approach as a new challenge for the future.

Primary malignant cardiac tumors represent (PMCTs) a very rare disease with an incidence of 0.009%1 (up to 10% of primary cardiac neoplasms) and are related to a very poor prognosis. The study by Mohamed Rahouma tries to give us information on sex differences in PMCTs, their incidence, behavior, and outcomes. Females were significantly older and had a lower stage of cancer. Males are known to have a more aggressive course and present at an earlier age. Sarcoma is the most common type of PMCTs in both males and females. There was no gender disparity in late mortality and patients who underwent surgery had a better prognosis than those who did not undergo surgery. Significant predictors of late mortality were found to be patients' high comorbidity index, angiosarcoma histology, and Stage III/IV. A challenge for cardiac surgeons is to improve survival in patients with cardiac malignancies, involving a multidisciplinary approach with oncologists, cardiologists, and radiologists. To pave the way for a significant improvement in survival in the future, more advanced sex-specific medical therapies for cancer such as novel chemotherapy agents, targeted immune therapies, genetic engineering need to be standardized to PMCTs and combined with radiological therapies such as gamma-knife and very advanced surgery to effectively treat even very aggressive forms of malignant tumors, with a significant impact on the patient's quality of life and survival.

Management of neonatal cardiac rhabdomyoma obstructing the aortic valve.

Rhabdomyomas associated with tuberous sclerosis are common cardiac tumors in children, and no surgical intervention is needed in most cases. However, when the tumor causes left ventricular outlet tract obstruction (LVOTO), immediate surgical intervention is indicated. Here, we report a newborn who was diagnosed antenatally with multiple cardiac rhabdomyomas, one of which blocked the aortic valve during systole. The tumor was resected early in the postnatal period with excellent outcomes. Until surgery, we maintained ductus arteriosus patency and systemic circulation using prostaglandin E1, which helped to reduce the risk of sudden death due to LVOTO. Postoperative two-dimensional echocardiography at discharge showed that surgery was effective in resolving LVOTO.

Sex differences in primary malignant cardiac tumors: A multi-institutional cohort study from National Cancer Database.

INTRODUCTION: Despite the significant clinical importance of sex among factors affecting cancer progression and survival, it remains one of the least studied factors. Therefore, we sought to examine these differences in relation to primary malignant cardiac tumors (PMCTs) using a national data set. METHODS: The 2004-2017 National Cancer Database was queried for patients with PMCTs. Annual trend of females' percent was assessed. Overall survival predictors were evaluated with Kaplan-Meier and Cox-regression. Subgroup analysis was done based on histology, comorbidity index, race, insurance, and surgical treatment. RESULTS: PMCTs were identified in 736 patients (median age 52, female [47.8%]). Most of them were high-grade (49.2%). About 60% underwent surgery. Angiosarcoma (43%), fibrosarcoma (5.2%), and leiomyosarcoma (5.2%) were the most common pathologies. Based on multivariate Cox-regression, higher income, higher comorbidity index, angiosarcoma, and Stage III/IV were associated with higher late mortality, while year of diagnosis and use of surgery or chemotherapy were associated with lower mortality. Among the surgical group, age, higher income, higher comorbidity index, angiosarcoma, and Stage III/IV were independent predictors of higher late mortality, while private insurance and year of diagnosis were associated with lower late mortality. No difference was seen between males and females in 30-day and late mortality (p = .71). Subgroup analysis based on Cox-regression showed no differences in late mortality between males and females. CONCLUSION: PMCTs have poor overall survival. Surgery and chemotherapy were associated with longer survival benefits. On the contrary, the associated risk factors for mortality were advanced age, higher comorbidity index, angiosarcoma histology, and Stage III/IV.

Differential diagnosis of cardiac tumors: General consideration and echocardiographic approach.

Cardiac tumors may be primary (either benign or malignant) or secondary (malignant) and are first detected by echocardiography in most cases. The cardiologist often challenges their identification, the differential diagnosis and the best therapeutic approach. Malignant tumors have usually a poor prognosis, which may be significantly improved by appropriate and timely therapies. The echocardiographic aspects of benign and malignant cardiac tumors described in this article, along with a clinical evaluation may orient the differential diagnosis and aid in choosing the further steps useful to define the nature of the mass.

miRNAs in Cardiac Myxoma: New Pathologic Findings for Potential Therapeutic Opportunities.

MicroRNAs (miRNAs) regulate gene expression at the post-transcriptional level, contributing to all major cellular processes. The importance of miRNAs in cardiac development, heart function, and valvular heart disease has been shown in recent years, and aberrant expression of miRNA has been reported in various malignancies, such as gastric cancer and breast cancer. Different from other fields of investigation, the role of miRNAs in cardiac tumors still remains difficult to interpret due to the scarcity publications and a lack of narrative focus on this topic. In this article, we summarize the available evidence on miRNAs and cardiac myxomas and propose new pathways for future research. miRNAs play a part in modifying the expression of cardiac transcription factors (miR-335-5p), increasing cell cycle trigger factors (miR-126-3p), interfering with ceramide synthesis (miR-320a), inducing apoptosis (miR-634 and miR-122), suppressing production of interleukins (miR-217), and reducing cell proliferation (miR-218). As such, they have complex and interconnected roles. At present, the study of the complete mechanistic control of miRNA remains a crucial issue, as proper understanding of signaling pathways is essential for the forecasting of therapeutic implications. Other types of cardiac tumors still lack adequate investigation with regard to miRNA. Further research should aim at investigating the causal relationship between different miRNAs and cell overgrowth, considering both myxoma and other histological types of cardiac tumors. We hope that this review will help in understanding this fascinating molecular approach.

Prenatally diagnosed large intrapericardial rhabdomyoma without haemodynamic compromise.

Cardiac rhabdomyoma is the most common primary cardiac tumour and is considered to be a hamartoma of developing embryonic cardiac myocytes. It is commonly seen in tuberous sclerosis. The size and location of the tumour is the surrogate determinant of the risk of haemodynamic compromise. Pericardial rhabdomyoma is very rare and tends to follow the inherent natural history of spontaneous regression. We present cases of two foetuses diagnosed with large pericardial rhabdomyoma and no haemodynamic consequences.

A Rare Case of Undifferentiated Pleomorphic Cardiac Sarcoma with Inflammatory Pattern.

Cardiac undifferentiated pleomorphic sarcoma (UPS) associated with fever and inflammatory response is an extremely rare condition. Herein, we report a rare case of cardiac UPS with unusual clinical presentation and inflammatory response. A 67-year-old male complaining of progressive dyspnea and intermittent fever of unknown cause was referred to our hospital for surgical resection of a left atrial mass. Laboratory analysis showed leukocytosis (26 × 103/µL) and high C-reactive protein (CRP) levels (155.4 mg/L). Hemoculture tests and urine analysis were negative for infection. A contrast chest computed tomography revealed a mass measuring 5.5 × 4.5 cm, occupying the left atrium cavity. The patient underwent surgical excision of the mass, however, surgical margin of the resected tumor could not be evaluated, due to the multifragmented nature of the resection specimen. Postoperative CRP and leukocyte levels normalized, highlighting the relationship between the tumor and the inflammatory status. Early diagnosis is crucial for a proper management and favorable outcome, enabling patients to undergo chemotherapy and achieve complete surgical resection.

Heart Failure Association, Heart Failure Society of America, and Japanese Heart Failure Society Position Statement on Endomyocardial Biopsy.

Endomyocardial biopsy (EMB) is an invasive procedure, globally most often used for the monitoring of heart transplant rejection. In addition, EMB can have an important complementary role to the clinical assessment in establishing the diagnosis of diverse cardiac disorders, including myocarditis, cardiomyopathies, drug-related cardiotoxicity, amyloidosis, other infiltrative and storage disorders, and cardiac tumors. Improvements in EMB equipment and the development of new techniques for the analysis of EMB samples has significantly improved the diagnostic precision of EMB. The present document is the result of the Trilateral Cooperation Project between the Heart Failure Association of the European Society of Cardiology, Heart Failure Society of America, and the Japanese Heart Failure Society. It represents an expert consensus aiming to provide a comprehensive, up-to-date perspective on EMB, with a focus on the following main issues: (1) an overview of the practical approach to EMB, (2) an update on indications for EMB, (3) a revised plan for heart transplant rejection surveillance, (4) the impact of multimodality imaging on EMB, and (5) the current clinical practice in the worldwide use of EMB.

Intracardiac echocardiographic-guided right-sided cardiac biopsy: Case series and literature review.

Endomyocardial biopsy (EMB) is a common procedure used to aid in the diagnosis of diffuse myocardial diseases and, less commonly, in the diagnosis of cardiac tumors. As cardiac tumors are often found in high-risk locations (ventricular free wall or atria), precision biopsy is paramount, and additional imaging, like transesophageal echocardiography is often required for guidance. The use of intracardiac echocardiography (ICE) to guide biopsy has been described, but there is no consensus on a standardized approach. We report our institutional approach with three cases of ICE-directed EMB performed with the 2.4 mm Jawz bioptome directed with an 8.5-Fr Agilis NxT steerable introducer. All cases were performed under guidance with the AcuNav ICE probe. There were no procedural complications and a definitive diagnosis was obtained in all three cases. We also review the available published cases of ICE-guided EMB in the literature-noting the different procedural approaches, complication rate, and diagnostic yield. There were only two negative biopsies reported among the published cases and no reported complications. Our review of all these cases suggests that ICE-guidance for EMB is superior to other forms of imaging in its ease of use and high definition of right-sided cardiac structures. We also feel that the use of the Agilis steerable sheath allows for more precise directing of the bioptome and is a critical component in performing a successful targeted biopsy.